Initial Visit Packet Forms

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1 NACC Uniform Data Set (UDS) FTLD Module Initial Visit Packet Forms Version 2.0, January 2012 Copyright 2013 University of Washington Created and published by the FTLD work group of the ADC Program (David Knopman, MD, Chair) and the National Alzheimer s Coordinating Center (Walter A. Kukull, PhD, Director). All rights reserved. This publication was funded by the National Institutes of Health through the National Institute on Aging (Cooperative Agreement U01 AG016976). NOTE: Version 2 is NOT the most current version of the FTLD Moudle forms and is no longer used for data submission. For the most current version, please visit This packet last modified December 13, 2013.

2 Guide to abbreviations AD ADC ADNI ALS bvftd CBD DLB FTLD FTLD Module IVP MCI MMSE MND NACC NIA PLS PPA SMA UDS UPDRS VaD Alzheimer s disease Alzheimer s Disease Center, any of 30 Centers across the United States participating in the Alzheimer s Disease Centers Program conducted by NIA Alzheimer s Disease Neuroimaging Initiative Amyotrophic lateral sclerosis Behavioral variant frontotemporal dementia Corticobasal degeneration Dementia with Lewy bodies Frontotemporal lobar degeneration A collection of data concerning FTLD on subjects in the NACC Uniform Data Set and appended to the UDS Initial Visit Packet, the set of forms completed at a subject s initial evaluation for submission to NACC Mild cognitive impairment Mini-mental state examination Motor neuron disease National Alzheimer s Coordinating Center, funded by NIA and charged with collecting data from the ADCs National Institute on Aging, one of the U.S. National Institutes of Health Primary lateral sclerosis Primary progressive aphasia Spinal muscular atrophy Uniform Data Set, the longitudinal database maintained by NACC; the other components of the NACC database are the Minimum Data Set (MDS) and the Neuropathologic Data Set (NP) Unified Parkinson s Disease Rating Scale Vascular dementia A note on form numbering Each NACC form has a unique two- to four-character number (e.g., B9, E2F, Z1, A3aF). For forms that are part of the FTLD Module to the UDS, the last character is F. As in the UDS, the first character of the form number indicates what kind of information is collected by the form: A: Family history (genetic) data B: Clinical data C: Neuropsychologic data E: Imaging data Z: Used only for the Form Checklist

3 Initial Visit Packet Form Z1F: Form Checklist NACC Uniform Data Set (UDS) ftld module NOTE: This form is to be completed by clinic staff. Examiner s initials: NACC expects and intends that all FTLD forms will be attempted on all subjects being evaluated for the FTLD Module of the UDS, but we realize this may be impossible when the subject is terminally ill, or when there is no informant, or for other reasons. Nevertheless, NACC requires that Forms B3F, B9F, C1F, C2F, C3F, E2F, and E3F be submitted for a subject to be included in the FTLD Module of the UDS database, even though these forms may include some missing data. For forms not designated as required, if it is not feasible to collect all or almost all of the data elements for a subject, and the ADC therefore decides not to attempt collection of those data, an explanation should be provided. Please indicate this decision by including the appropriate explanatory code and any additional comments. KEY: If the specified form was not completed, please enter one of the following codes: 95=Physical problem 96=Cognitive/behavior problem 97=Other problem 98=Verbal refusal 99=Unknown or inadequate information Form Description Submitted: Yes No If not submitted, specify reason (see key, above) Comments (provide if needed) A3aF Record of Consent for Biologic Specimen Use 1 0 A3F Family History: Affected Family Members 1 0 B3F Supplemental UPDRS Required B9F Clinical PPA and bvftd Features Required C1F Neuropsychological Battery Summary Scores Required C2F Social Norms Questionnaire Required C3F Social Behavior Observer Checklist Required C4F Behavioral Inhibition Scale 1 0 C5F Interpersonal Reactivity Index 1 0 C6F Revised Self-monitoring Scale 1 0 E2F Imaging Available Required E3F Imaging in Diagnosis Required UDS/FTLD Version 2.0, January 2012 Page 1 of 1

4 INITIAL Visit Packet NACC Uniform Data Set (UDS) ftld module Form A3aF: Record of Consent for Biologic Specimen Use NOTE: This form is to be completed by clinic staff responsible for obtaining consents, based on an existing consent at clinic. For additional clarification and examples, see FTLD Coding Guidebook for Initial Visit Packet, Form A3aF. Examiner s initials: One of these forms will be completed for each relative who provides a specimen. 1. What relative s consent is being recorded on this form? NOTE: Unknown (9999) is not a permissible value for sibling s or child s birth year. If birth year is unknown, please provide an approximate year on UDS Initial Visit Form A3 so that the sibling or child ends up in correct birth order relative to the other siblings or children. Sibling s birth year or child s birth year on this form MUST agree with the birth year listed for that person on UDS Initial Visit or UDS Follow-up Visit Form A3 and FTLD Module Initial Visit or FTLD Follow-up Visit Form A3F (if applicable) Mother Father Sibling (sibling s birth year: ) Child (child s birth year: ) Please indicate that the above relative provided consent for the following. The wording need not be identical but should explicitly express the same points. 1a. I permit my sample to be stored and used in future research of neurologic disease at (home institution). 1b. I permit my sample to be stored and used in future research at (home institution) to learn about, prevent, or treat other health problems. 1c. There is a small chance that some commercial value may result from my sample at the National Cell Repository for Alzheimer s Disease (NCRAD). If that would happen, I would not be offered a share in any profits. I permit (home institution) to give my sample to researchers at other institutions. 0 No 1 Yes 0 No 1 Yes 0 No 1 Yes UDS/FTLD Version 2.0, January 2012 Page 1 of 1

5 Initial Visit Packet NACC Uniform Data Set (UDS) ftld module Form A3F: Family History: Affected Family Members NOTE: This form is to be completed by a clinician with experience in evaluating patients with frontotemporal lobar degeneration. For additional clarification and examples, see FTLD Coding Guidebook for Initial Visit Packet, Form A3F. Examiner s initials: AFFECTED FAMILY MEMBERS Please consider blood relatives only. For the purposes of Form A3F, affected means affected by dementia OR by any of the non-normal clinical diagnoses listed in Appendix 1 on page 4 of this form. AFFECTED FAMILY MEMBERS 1a. Are there affected family members? (See box above for definition of affected. ) If the answer is No or Unknown, please skip the rest of this form. 0 No 1 Yes 9 Unknown 1b. In this family, is there a known mutation in a gene associated with FTLD? If the answer is No or Unknown, please skip to Question 2. 0 No 1 Yes 9 Unknown 1c. What is the predominant mutation? 1 MAPT 2 PGRN 3 C9ORF72 4 FUS 8 Other (SPECIFY: ) 9 Unknown 1d. Is there evidence for this mutation in the form of commercial lab test documentation? 1e. Is there evidence for this mutation in the form of research lab test documentation? 1f. Is there evidence for this mutation in the form of family report? 1g. Is there other evidence for this mutation? 0 No 1 Yes 9 Unknown 0 No 1 Yes 9 Unknown 0 No 1 Yes 9 Unknown 0 No 1 Yes (SPECIFY: ) 9 Unknown UDS/FTLD Version 2.0, January 2012 Page 1 of 4

6 AFFECTED PARENTS Use the form below to provide information on affected parents only (see definition of affected in the box above). AFFECTED PARENTS 2a. Mother 2b. Father AFFECTED SIBLINGS Use the form below to provide information on affected siblings only (see definition of affected in the box on page 1 of this form). Sibling s birth year on this form MUST agree with the birth year listed for that sibling on UDS Initial Visit or UDS Follow-up Visit Form A3 and FTLD Module Initial Visit or FTLD Follow-up Visit Form A3aF (if applicable). Unknown (9999) is not a permissible value. If birth year is unknown, please provide an approximate year on UDS Initial Visit Form A3 so that the sibling with unknown birth year ends up in correct birth order relative to the other siblings. (EXAMPLE: Suppose a subject is the oldest of three children. The subject was born in 1930 and the middle sibling in 1933; the youngest sibling s birth year is unknown. An approximate birth year of 1934 or later should be assigned to the youngest sibling.) Use that same birth year on FTLD Module Forms A3F and A3aF. If an affected sibling has already been listed on UDS Initial Visit Form A3 with a birth year of 9999, then UDS Initial Visit Form A3 must be edited so that an approximate birth year is entered, as described in the paragraph above. That same birth year should be entered below. Sibling s birth month should be filled out if known; otherwise, please enter 99. Only full siblings should be listed. AFFECTED SIBLINGS 3a. / 3b. / 3c. / 3d. / 3e. / 3f. / 3g. / 3h. / 3i. / 3j. / 3k. / 3l. / 3m. / a. Neurological problem* a. Sibling s birth mo / yr b. Primary DX** b. Neurological problem* c. Method of evaluation*** c. Primary DX** d. Age of onset d. Method of evaluation*** e. Age of onset *Codes for neurological problems and psychiatric conditions 1 Cognitive impairment/ behavior change 2 Parkinsonism 3 ALS 4 Other neurologic condition such as multiple sclerosis or stroke 5 Psychiatric condition such as schizophrenia, bipolar disorder, or alcoholism 9 Unknown **Codes for primary diagnosis See Appendix 1 on page 4 of this form ***Codes for method of evaluation For descriptions, see Appendix 2 on page 4 of this form 1 Autopsy 2 Examination 3 Medical record review from formal dementia evaluation 4 Review of general medical records AND informant and/or subject telephone interview 5 Review of general medical records only 6 Subject and/or informant telephone interview 7 Family report Initial Visit Form A3F: Family History: Affected Members (UDS/FTLD Version 2.0, January 2012) Page 2 of 4

7 AFFECTED CHILDREN Use the form below to provide information on affected children only (see definition of affected in the box on page 1 of this form). Child s birth year on this form MUST agree with the birth year listed for that child on UDS Initial Visit or UDS Follow-up Visit Form A3 and FTLD Module Initial Visit or FTLD Follow-up Visit Form A3aF (if applicable). Unknown (9999) is not a permissible value. If birth year is unknown, please provide an approximate year on UDS Initial Visit Form A3 so that the child with unknown birth year ends up in correct birth order relative to the other children. (EXAMPLE: Suppose a subject has three children. The oldest is a son born in 1960, the youngest a son born in 1964, and the middle child a girl whose birth year is unknown. The girl should be assigned an approximate birth year of 1962 or 1963.) Use that same birth year from UDS Initial Visit Form A3 on FTLD Module Forms A3F and A3aF. If an affected child has already been listed on UDS Initial Visit Form A3 with a birth year of 9999, then UDS Initial Visit Form A3 must be edited so that an approximate birth year is entered, as described in the paragraph above. That same birth year should be entered below. Child s birth month should be filled out if known; otherwise, please enter 99. AFFECTED CHILDREN 4a. / 4b. / 4c. / 4d. / 4e. / 4f. / 4g. / 4h. / 4i. / 4j. / 4k. / a. Child s birth mo / yr b. Neurological problem* c. Primary DX** d. Method of evaluation*** e. Age of onset *Codes for neurological problems and psychiatric conditions 1 Cognitive impairment/ behavior change 2 Parkinsonism 3 ALS 4 Other neurologic condition such as multiple sclerosis or stroke 5 Psychiatric condition such as schizophrenia, bipolar disorder, or alcoholism 9 Unknown **Codes for primary diagnosis See Appendix 1 on page 4 of this form ***Codes for method of evaluation For descriptions, see Appendix 2 on page 4 of this form 1 Autopsy 2 Examination 3 Medical record review from formal dementia evaluation 4 Review of general medical records AND informant and/or subject telephone interview 5 Review of general medical records only 6 Subject and/or informant telephone interview 7 Family report 4l. / 4m. / Initial Visit Form A3F: Family History: Affected Members (UDS/FTLD Version 2.0, January 2012) Page 3 of 4

8 **APPENDIX 1: PRIMARY DIAGNOSIS CODES CODE DIAGNOSIS 040 Mild cognitive impairment (MCI), not otherwise specified 041 MCI amnestic 042 MCI multiple domain with amnesia 043 MCI single domain nonamnestic 044 MCI multiple domain nonamnestic 045 Impaired, but not MCI 050 Alzheimer s disease 070 Dementia with Lewy bodies 080 Vascular dementia 100 Alcohol-related dementia 110 Dementia of undetermined etiology 120 Behavioral variant frontotemporal dementia 130 Primary progressive aphasia, semantic variant 131 Primary progressive aphasia, nonfluent/agrammatic variant 132 Primary progressive aphasia, logopenic variant 133 Primary progressive aphasia, not otherwise specified CODE DIAGNOSIS 140 Progressive supranuclear palsy 150 Corticobasal syndrome/corticobasal degeneration 160 Huntington s disease 170 Prion disease 180 Cognitive dysfunction from medications 190 Cognitive dysfunction from medical illness 200 Depression 210 Other major psychiatric illness 220 Down syndrome 230 Parkinson disease 240 Stroke 250 Hydrocephalus 260 Traumatic brain injury 270 CNS neoplasm 280 Other 310 Amyotrophic lateral sclerosis 320 Multiple sclerosis ***APPENDIX 2: METHOD OF EVALUATION 1. Autopsy If the autopsy was performed at an outside institution, you must have the report to code as diagnosis by autopsy. 2. Examination The subject must have been examined in person at your ADC/institution or by genetic studies staff associated with your ADC/institution to code as diagnosis by examination. Medical records may or may not have been used when assigning diagnosis. 3. Medical record review from formal dementia evaluation Medical records should be from an examination that focused specifically on dementia; that was performed by a neurologist, geriatrician, or psychiatrist; that includes a neurologic examination, an imaging study, and cognitive testing (e.g., MMSE, Blessed, or more formal tests). A telephone interview may also be used to collect additional information. 4. Review of general medical records AND informant and/or subject telephone interview General medical records can be of various types, including those from a primary-care physician s office, hospitalization records, nursing home records, etc. They may include a neurologic exam and a cognitive test such as the MMSE along with a medical history. The telephone interview with the subject and/or the informant should include a medical history to capture the nature and presentation of cognitive deficits, if present, and age of onset if symptomatic. If the subject is normal or is in the early stages of dementia, brief formal cognitive testing should be included in the interview. Unless an affected subject is in the early stages of dementia, the interview should be conducted with an informant. 5. Review of general medical records ONLY See definition No. 4 above. If general medical records are used to diagnose a subject as demented or not demented, they should include a medical history, neurologic exam, and a cognitive test such as an MMSE. In most cases, general medical records alone should not be used to assign a diagnosis of mild cognitive impairment, or of any of the FTLD spectrum subtypes, or of parkinsonian disorders other than Parkinson disease. 6. Subject and/or informant telephone interview See definition No. 4 above. 7. Family report Family report should be coded when the informant for the family reports a subject as having been diagnosed with a particular disorder. In most cases, family report alone should not be used to assign a diagnosis of mild cognitive impairment, or of any of the FTLD spectrum subtypes, or of parkinsonian disorders other than Parkinson disease. Initial Visit Form A3F: Family History: Affected Members (UDS/FTLD Version 2.0, January 2012) Page 4 of 4

9 Initial Visit Packet NACC Uniform Data Set (UDS) ftld module Form B3F: Supplemental UPDRS NOTE: This form is to be completed by the clinician or other trained health professional. For additional clarification and examples, see FTLD Module Coding Guidebook for Initial Visit Packet, Form B3F. Check only one box per question. Examiner s initials: Not to a degree that would justify such a diagnosis Yes with asymmetry L>R R>L Yes without major asymmetry Untestable SECTION A A1. Does the subject have limb or torso fasciculations consistent with a diagnosis of spinal muscular atrophy (SMA) or amyotrophic lateral sclerosis (ALS)*? A2. Does the subject have limb weakness and/or hyperreflexia consistent with a diagnosis of primary lateral sclerosis (PLS) or ALS*? A3. Does the subject have bulbar weakness and/or fasciculations consistent with a diagnosis of ALS*? A4. Does the subject have eye movement abnormalities consistent with a diagnosis of progressive supranuclear palsy (PSP)*? A5. Does the subject have dystonia* or rigidity consistent with a diagnosis of corticobasal degeneration (CBD)*? A6. Is there ideomotor apraxia* consistent with a diagnosis of CBD? A7. Is the alien limb phenomenon* present consistent with a diagnosis of CBD? A8. Is there myoclonus* consistent with a diagnosis of CBD? A9. Is there a cortical sensory deficit* consistent with a diagnosis of CBD? *NOTE: For the items marked with an asterisk, definitions and explanations can be found in the FTLD Module Coding Guidebook for Initial Visit Packet, Form B3F. UDS/FTLD Version 2.0, January 2012 Page 1 of 2

10 SECTION B Gait disturbances B1. Severity Normal Slight alteration in speed or fluidity of gait Walks with difficulty but requires no assistance Severe disturbance Cannot walk at all Untestable (specify reason): B2. Type Normal Hemiparetic (spastic) Foot drop gait (lower motor neuron) Ataxic gait Parkinsonian gait Apractic gait ( magnetic gait ) Antalgic gait Other gait disorder not listed above (specify): Untestable (specify reason): Initial Visit Form B3F: Supplemental UPDRS (UDS/FTLD Version 2.0, January 2012) Page 2 of 2

11 Initial Visit Packet NACC Uniform Data Set (UDS) ftld module Form B9F: Clinical PPA and bvftd Features NOTE: This form is to be completed by a clinician with experience in evaluating subjects with frontotemporal lobar degeneration. For additional clarification, see FTLD Coding Guidebook for Initial Visit Packet, Form B9F. Check only one box per question. Examiner s initials: Gateway question for primary progressive aphasia (PPA) No Yes 1. Does the subject have an acquired and progressive difficulty with language* consistent with PPA of a neurodegenerative type? *DIFFICULTY WITH LANGUAGE: Other than simple dysarthria, are there difficulties with retrieving, using, repeating, sequencing, or understanding words? If answer is No, check 0 (Absent) for Questions 2 11 and 0 (No) for Question Characterizing speech and language symptoms / assigning PPA subtype Are these features present on the current examination? Note: many of these items are also evaluated in the neuropsychological assessment. The responses recorded here should represent the consensus of the clinical and neuropsychological evaluation. Absent Questionably present Definitely present Not evaluated 2. Poor object naming (Core diagnostic feature of semantic variant; abnormal in all variants) 3. Impoverished word selection / retrieval in spontaneous speech or writing (Core diagnostic feature of logopenic variant; abnormal in all variants) Impaired word comprehension (Core diagnostic feature of semantic variant; absent in other variants) Poor object/person knowledge (Secondary diagnostic feature of semantic variant; absent in other variants) Grammatical simplification or grammatical errors in speech or writing (Core diagnostic feature of nonfluent/agrammatic variant) Effortful, halting speech (Core diagnostic feature of nonfluent/agrammatic variant) UDS/FTLD Version 2.0, January 2012 Page 1 of 4

12 Characterizing speech and language symptoms / assigning PPA subtype (continued) Absent Questionably present Definitely present Not evaluated 8. Circumlocutory, empty speech (Secondary diagnostic feature of logopenic variant; also present in semantic variant) Speech sound/word errors (paraphasias) (Secondary diagnostic feature of logopenic variant; abnormal in nonfluent/ agrammatic variant) Impaired speech repetition (inability to repeat verbatim sentence-length material) (Core diagnostic feature of logopenic variant; present in nonfluent/agrammatic type; absent in semantic variant) Surface dyslexia and dysgraphia also refer to Word Reading Test from FTLD Neuropsychological Battery (Secondary feature of semantic variant) 12. ROOT DIAGNOSIS OF PPA Does the subject have an acquired and progressive difficulty with language consistent with PPA of a neurodegenerative type AND is the language disorder the most prominent deficit at symptom outset and for the initial phase (1 2 years) of the disorder? 0 No Proceed to Question 14 1 Yes Meets root diagnosis of PPA Proceed to Question Consensus diagnosis of dominant PPA subtype based on clinician and neuropsychologist judgment NOTE: The diagnostic criteria in this module do not match the criteria in UDS V2.0 (Form D1). While Version 2.0 of the UDS is still in use, keep the two sets of diagnostic criteria separate. 1 PPA, semantic variant (semppa) 2 PPA, nonfluent/agrammatic variant (nf/gppa) 3 PPA, logopenic variant 4 PPA not otherwise specified Initial Visit Form B9F: Clinical PPA and bvftd Features (UDS/FTLD Version 2.0, January 2012) Page 2 of 4

13 Gateway question for behavioral variant frontotemporal dementia (bvftd) No Yes 14. Does the subject have acquired, clinically important alterations in behavior, personality, or comportment consistent with bvftd of a neurodegenerative type? If answer is No, check 0 (Absent) for Questions and 0 (Meets <3 of the features described in Questions 15 21: does not meet criteria for bvftd; or an exclusionary feature is present.) for Question Characterizing symptoms of bvftd Have the following symptoms/behaviors been prominent, persistent, and recurrent in (approximately) the past three years? Absent Questionably present Definitely present Not evaluated 15. Disinhibition Socially inappropriate behavior; loss of manners or decorum; impulsive, rash, or careless actions Apathy or inertia Loss of interest, drive, and motivation; decreased initiation of behavior loss of sympathy / empathy Diminished response to other people s needs or feelings; diminished social interest, interrelatedness, or personal warmth Ritualistic / compulsive behavior Simple repetitive movements or complex compulsive or ritualistic behaviors Hyperorality and appetite changes Altered food preferences, binge eating, increased consumption of alcohol or cigarettes, oral exploration or consumption of inedible objects Changes on neuropsychological testing consistent with bvftd (refer to neuropsychological evaluation and neuropsychologist s impression) Impaired daily functioning Are these alterations in behavior, personality, or comportment the principal cause of impaired daily living activities? Initial Visit Form B9F: Clinical PPA and bvftd Features (UDS/FTLD Version 2.0, January 2012) Page 3 of 4

14 22. Does the subject meet the criteria for clinical probable* or possible** bvftd syndrome? *PROBABLE: Meets three of the above criteria and has impaired daily functioning and has imaging consistent with bvftd. **POSSIBLE: Meets three of the above criteria but is not functionally impaired or does not having imaging consistent with bvftd. NOTE: The diagnostic criteria in this module do not match the criteria in UDS V2.0 (Form D1). While Version 2.0 of the UDS is still in use, keep the two sets of diagnostic criteria separate Meets <3 of the features described in Questions 15 21: does not meet criteria for bvftd; or an exclusionary feature is present. Probable bvftd. Meets criteria for possible bvftd and has impaired daily functioning but without evidence of diagnostic imaging. Meets criteria for possible bvftd (with or without evidence of diagnostic imaging), but daily functioning is not significantly impaired. No Yes Uncertain 23. Was an electromyogram (EMG) performed at this visit? If answer is 1 (Yes), SKIP TO QUESTION Has an EMG been performed in the past year? If answer is 0 (No), SKIP TO QUESTION If an EMG was performed, did it show evidence of motor neuron disease? If subject has only one diagnosis (either PPA or bvftd), then END FORM HERE. 26. For subjects with a diagnosis of both PPA and bvftd, which diagnosis appeared first? 1 bvftd 2 PPA, semantic variant 3 PPA, nonfluent/agrammatic variant 4 PPA, logopenic variant 5 PPA not otherwise specified 9 Unknown Initial Visit Form B9F: Clinical PPA and bvftd Features (UDS/FTLD Version 2.0, January 2012) Page 4 of 4

15 initial Visit Packet NACC Uniform Data Set (UDS) ftld module Form C1F: Neuropsychological Battery Summary Scores NOTE: This form is to be completed by ADC or clinic staff. For test administration and scoring, see FTLD Module Coding Guidebook for Initial Visit Packet, Form C1F. Time to completion of C1F and C2F tests should be reported at the end of form C3F. Examiner s initials: KEY: If the subject cannot complete any of the following exams, please give the reason by entering one of the following codes in the first data element and skip the rest of the data elements for that test: 95 = Physical problem 96 = Cognitive/behavior problem 97 = Other problem 98 = Verbal refusal 1. Benson Complex Figure Copy 1a. Total score for copy of Benson figure (0 17) Verbal Fluency: Phonemic Test 2a. Number of correct F-words generated in 1 minute (0 40)... 2b. Number of F-words repeated in 1 minute (0 15)... 2c. Number of non-f-words and rule violation errors in 1 minute (0 15)... 2d. Number of correct L-words generated in 1 minute (0 40)... 2e. Number of L-words repeated in one minute (0 15).... 2f. Number of non-l-words and rule violation errors in 1 minute (0 15)... 2g. TOTAL number of correct F-words and L-words (0 80)... 2h. TOTAL number of F-word and L-word repetition errors (0 30)... 2i. TOTAL number of non-f/l words and rule violation errors (0 30)... UDS/FTLD Version 2.0, January 2012 Page 1 of 3

16 KEY: 95 = Physical problem 96 = Cognitive/behavior problem 97 = Other problem 98 = Verbal refusal 3. Word Reading Test Regular and Irregular Words REGULAR 3a. Total completely accurate words (0 15)... 3b. Total semantically related inaccurate words (0 15)... 3c. Total other phonologically related words or nonword errors (0 15)... IRREGULAR 3d. Total completely accurate words (0 15)... 3e. Total semantically related inaccurate words (0 15)... 3f. Total words that are regularized (read using phonics, e.g., sew read as sue) (0 15).. 3g. Total other phonologically related words or nonword errors (0 15) Benson Complex Figure Delay (Recall) 4a. Total score for 10- to 15-minute delayed drawing of Benson figure (0 17) b. Recognized original stimulus from among four options?... 0 No 1 Yes 5. Semantic Word-picture Matching Test 5a. Total correct word-picture matches (0 20) Semantic Associates Test 6a. Total correct animal associations (0 8)... 6b. Total correct tool associations (0 8)... 6c. Sum of all correct associations (Semantic Associates Test total score) (0 16) Northwestern Anagram Test Short Form 7a. Correct subject who-questions (0 5)... 7b. Correct object who-questions (0 5)... 7c. Total score: sum of all correct questions (0 10)... Initial Visit Form C1F: Neuropsych Summary Scores (UDS/FTLD Version 2.0, January 2012) Page 2 of 3

17 KEY: 95 = Physical problem 96 = Cognitive/behavior problem 97 = Other problem 98 = Verbal refusal 8. Sentence Repetition Test 8a. Number of completely accurate sentences (0 5).... 8b. Total number of words omitted from sentences (0 37).... 8c. Total number of semantically related or unrelated incorrect real words (0 20)... 8d. Total number of phonologically related words or nonword errors (0 20) Noun and Verb Naming Subtests 9a. Total nouns correct (0 16)... 9b. Total verbs correct (0 16).... 9c. Noun-to-verb ratio (total nouns correct / total verbs correct) Sentence Reading Test 10a. Number of completely accurate sentences (0 5) b. Total number of words omitted from sentence (0 37)... 10c. Total number of semantically related or unrelated incorrect real words (0 20)... 10d. Total number of phonologically related words or nonword errors (0 20)... Initial Visit Form C1F: Neuropsych Summary Scores (UDS/FTLD Version 2.0, January 2012) Page 3 of 3

18 Initial Visit Packet NACC Uniform Data Set (UDS) ftld module Form C2F: Social Norms Questionnaire 1 INSTRUCTIONS FOR THE CENTER This questionnaire is designed to be completed by the subject in the presence of a qualified psychologist or psychometrist as part of a face-to-face battery of tests. The examiner should read and explain the instructions to the subject, then ask the subject to complete the questionnaire. If the subject asks for clarification of the procedure or questions, it is acceptable for the examiner to discuss the questionnaire with him or her. Tell the subject that Don t know and Not applicable are not allowable responses for any item. While it is permissible to help a cognitively impaired subject understand and complete the questionnaire (e.g., by reading the questions out loud, or marking their response for them), the examiner should ensure that they merely help the subject understand a question (e.g., by saying, Do you think it s OK to cut in line if you are in a hurry? ), but not help them formulate their response. In this way, if the subject asks what they should answer, it would be permissible to respond with prompts such as, It s up to you. Answer whatever you think is best. It s OK to guess if you re not sure. KEY: If the subject is so impaired as to make administration of this questionnaire impossible, please give the reason by checking one of the following reason codes in the FOR CLINIC USE ONLY section and skip the remaining data elements. 95 = Physical problem 96 = Cognitive/behavior problem 97 = Other problem 98 = Verbal refusal If the subject completes some but not all of the questionnaire, items that are missing should be left blank, and all affected summary scores should be entered as 88 or 88.88, as appropriate. 1 Reproduced by permission of the author, Katherine Rankin, PhD; do not copy or distribute without author s permission. Form created as part of the FTLD Module to the National Alzheimer s Coordinating Center Uniform Data Set, copyright 2013 University of Washington.the FTLD Module to the National Alzheimer s Coordinating Center Uniform Data Set, copyright 2013 University of Washington. UDS/FTLD Version 2.0, January 2012

19 Initial Visit Packet NACC Uniform Data Set (UDS) ftld module Form C2F: Social Norms Questionnaire 1 FOR CLINIC USE ONLY: Reason code: 95=Physical problem 96=Cognitive/behavior problem 97=Other problem 98=Verbal refusal Examiner s initials: Instructions: Following is a list of behaviors that a person might engage in. Please decide whether or not it would be socially acceptable and appropriate to do these things in the mainstream culture of the United States and answer yes or no to each. Think about these questions as if they were occurring in front of or with a stranger or acquaintance, NOT a close friend or family member. Would it be socially acceptable to: 1. Tell a stranger you don t like their hairstyle? 0 NO 1 YES 2. Spit on the floor? 0 NO 1 YES 3. Blow your nose in public? 0 NO 1 YES 4. Ask a coworker their age? 0 NO 1 YES 5. Cry during a movie at the theater? 0 NO 1 YES 6. Cut in line if you are in a hurry? 0 NO 1 YES 7. Laugh when you yourself trip and fall? 0 NO 1 YES 8. Eat pasta with your fingers? 0 NO 1 YES 9. Tell a coworker your age? 0 NO 1 YES 10. Tell someone your opinion of a movie they haven t seen? 0 NO 1 YES 11. Laugh when someone else trips and falls? 0 NO 1 YES 12. Wear the same shirt every day? 0 NO 1 YES 1 Reproduced by permission of the author, Katherine Rankin, PhD; do not copy or distribute without author s permission. Form created as part of the FTLD Module to the National Alzheimer s Coordinating Center Uniform Data Set, copyright 2013 University of Washington.the FTLD Module to the National Alzheimer s Coordinating Center Uniform Data Set, copyright 2013 University of Washington. UDS/FTLD Version 2.0, January 2012 Page 1 of 2

20 13. Keep money you find on the sidewalk? 0 NO 1 YES 14. Pick your nose in public? 0 NO 1 YES 15. Tell a coworker you think they are overweight? 0 NO 1 YES 16. Eat ribs with your fingers? 0 NO 1 YES 17. Tell a stranger you like their hairstyle? 0 NO 1 YES 18. Wear the same shirt twice in two weeks? 0 NO 1 YES 19. Tell someone the ending of a movie they haven t seen? 0 NO 1 YES 20. Hug a stranger without asking first? 0 NO 1 YES 21. Talk out loud during a movie at the theater? 0 NO 1 YES 22. Tell a coworker you think they have lost weight? 0 NO 1 YES 1 Reproduced by permission of the author, Katherine Rankin, PhD; do not copy or distribute without author s permission. Form created as part of the FTLD Module to the National Alzheimer s Coordinating Center Uniform Data Set, copyright 2013 University of Washington.the FTLD Module to the National Alzheimer s Coordinating Center Uniform Data Set, copyright 2013 University of Washington. FOR CLINIC USE ONLY. Note: Calculation of the four summary scores below is OPTIONAL. 23. SNQ22 Total Score (0 22): 24. Break Score (0 12): 25. Overadhere Score (0 10): 26. Yes-No Ratio Score (0 22):. Initial Visit Form C2F: Social Norms Questionnaire (UDS/FTLD Version 2.0, January 2012) Page 2 of 2

21 Initial Visit Packet NACC Uniform Data Set (UDS) ftld module Form C3F: Social Behavior Observer Checklist 1 NOTE: This form is to be completed by the examiner who administered the neuropsychological battery to the subject. For additional clarification and examples, see FTLD Module Instructions for Neuropsychological Questionnaires (Forms C2F C6F) and Tests Reported on Form C1F. Check only one box per question. Examiner s initials: Directions: Immediately after the end of your evaluation of the subject, please rate his/her behavior during the time he/she was with you. Use the scales for both the main descriptors (i.e., 1, 2, 3 ) and the behavior counts (a., b., c. ) and complete all items. Your descriptor ratings and behavior counts for the same item can be independent. You may describe the subject as having a particular characteristic on a main descriptor, even if you endorse never for all of the behavior counts for that item, or vice versa. 1. Was overly self-conscious / embarrassed for self: 0 Not at all 1 A little bit 2 Moderately 3 Severely a. Spontaneously mentioned that he/she was performing badly b. Made other self-depreciatory comments c. Showed emotional distress over his/her performance / cognitive abilities 2. Showed too little self-consciousness / embarrassment for self: a. Disrobed immodestly (took off shoes, belt, pants, etc.; lifted shirt, etc.) b. Engaged in belching or flatulence, or picked nose without apology c. Giggled or otherwise made silly, childish comment or noise 0 Not at all 1 A little bit 2 Moderately 3 Severely 3. Insensitive to others embarrassment or privacy: 0 Not at all 1 A little bit 2 Moderately 3 Severely a. Insulted or made a negative comment about examiner b. Made an embarrassing comment about examiner c. Made an inappropriate or embarrassing joke 1 Reproduced by permission of the author, Katherine Rankin, PhD; do not copy or distribute without author s permission. Form created as part of the FTLD Module to the National Alzheimer s Coordinating Center Uniform Data Set, copyright 2013 University of Washington. UDS/FTLD Version 2.0, January 2012 Page 1 of 3

22 4. Failed to adapt / defer to structure of testing situation established by examiner: a. Resisted redirection while engaging in a verbal monologue 0 Not at all 1 A little bit 2 Moderately 3 Severely b. Interrupted examiner c. Verbalized a desire to leave the evaluation prematurely d. Physically attempted to leave the evaluation prematurely e. Failed to maintain topic of discussion, initiated tangent f. Demanded that test protocol be broken for him/her (e.g., insisted on completing an item after being told to stop, tried to cheat, tried to turn page to advance to next item against examiner s expressed wishes, etc.) 5. Was preoccupied with time or kept a strict timetable: a. Reminded examiner what time evaluation had to be finished 0 Not at all 1 A little bit 2 Moderately 3 Severely 6. Acted overly dependent: 0 Not at all 1 A little bit 2 Moderately 3 Severely a. Mentioned caregiver s absence or asked when caregiver would return b. Asked for feedback on performance c. Showed frustration when examiner would not provide explicit feedback 7. Was anxious: 0 Not at all 1 A little bit 2 Moderately 3 Severely a. Mentioned being nervous about testing / performance anxiety b. Mentioned being nervous about diagnosis or prognosis 8. Was stimulus-bound: 0 Not at all 1 A little bit 2 Moderately 3 Severely a. Made stimulus-bound error on testing b. Picked up object on desk unnecessarily c. Circumstantial speech; overly focused on details, overly lengthy 9. Was perseverative: 0 Not at all 1 A little bit 2 Moderately 3 Severely a. Repeated previous answer on testing b. Repeated an anecdote Initial Visit Form C3F: Social Behavior Observer Checklist (UDS/FTLD Version 2.0, January 2012) Page 2 of 3

23 10. Showed decreased initiation: 0 Not at all 1 A little bit 2 Moderately 3 Severely a. Began response in a notably delayed manner (not due to general slowing) b. Required additional verbal prompting to initiate task 11. Showed fluctuating level of cognitive ability through sessions regardless of complexity of material (e.g., was coherent at times and had notable difficulty understanding at other times): a. Lost task set / forgot instructions after performing task correctly b. Repeated rules to self multiple times during task c. Lost train of thought during conversation or response (demonstrated thought blocking) 0 Not at all 1 A little bit 2 Moderately 3 Severely 12. Was overly disclosing or inappropriately familiar: 0 Not at all 1 A little bit 2 Moderately 3 Severely a. Spontaneously revealed inappropriately personal information concerning self (only) b. Spontaneously revealed inappropriately personal information concerning a relative or friend (can also involve self) c. Stood or leaned too close to examiner (noticeably entered examiner s personal space) d. Touched examiner 13. Showed diminished social / emotional engagement: 0 Not at all 1 A little bit 2 Moderately 3 Severely 14. Showed exaggerated / labile emotional reactivity: 0 Not at all 1 A little bit 2 Moderately 3 Severely 15. DESCRIPTOR TOTAL SCORE (0 42): 16. CHECKLIST (BEHAVIOR) SCORE (0 105): 17. Length of the entire FTLD neuropsychological testing session: Record in minutes the approximate length of the testing session upon which these checklist responses were based. This should include, at minimum, time spent on all tests in the FTLD neuropsychological battery (all tests recorded on Form C1F, plus Form C2F), as well as time spent administering any other neuropsychological tests. Rankin (2010) 1 Reproduced by permission of the author, Katherine Rankin, PhD; do not copy or distribute without author s permission. Form created as part of the FTLD Module to the National Alzheimer s Coordinating Center Uniform Data Set, copyright 2013 University of Washington. Initial Visit Form C3F: Social Behavior Observer Checklist (UDS/FTLD Version 2.0, January 2012) Page 3 of 3

24 Initial Visit Packet NACC Uniform Data Set (UDS) ftld module Form C4F: Behavioral Inhibition Scale 1 Informant Questionnaire INSTRUCTIONS FOR THE CENTER This questionnaire is designed to be completed independently by the informant, who will be describing the subject s current typical behavior. This form may be handed to the informant for completion by him- or herself at any time during the study visit. Tell the subject that Don t know and Not applicable are not allowable responses for any item. If the informant asks for clarification of questions, it is acceptable for a qualified psychologist or psychometrist to discuss the questionnaire with him or her. However, if the informant completes this questionnaire collaboratively with the clinician, either face-to-face or via telephone, you must inform NACC of this change in protocol by checking the appropriate box in the gray FOR CLINIC USE ONLY area at the top of the questionnaire. Before the informant leaves, clinic staff should make sure that all questions were completed by the informant (i.e., none was left blank) by discussing the missing item with the informant and encouraging them to provide a response. If this is not done and it is later noticed that some items were missed by the informant, clinic staff should call the informant as soon as possible so that the missing items can be completed by phone. In this case, the questionnaire is not considered to have been completed independently by the informant. In the shaded area at the top of the form, the appropriate response would therefore be, This questionnaire was completed via telephone interview of informant by clinic staff. If there are still missing items, these items should be left blank, and 88 should be entered for the total score. Copyright 1994 by the American Psychological Association. Adapted with permission. The official citation that should be used in referencing this material is Table 1 (adapted), p. 323, from Carver C. S., & White, T. L. (1994). Behavioral inhibition, behavioral activation, and affective responses to impending reward and punishment: The BIS/BAS Scales. Journal of Personality and Social Psychology, 67(2), doi: / No further reproduction or distribution is permitted without written permission from the American Psychological Association. UDS/FTLD Version 2.0, January 2012

25 Initial Visit Packet NACC Uniform Data Set (UDS) ftld module Form C4F: Behavioral Inhibition Scale 1 Informant Questionnaire FOR CLINIC USE ONLY: This questionnaire was completed: 0 Independently by informant, as described in Instructions to the Center 1 Via in-person interview of informant by clinic staff 2 Via telephone interview of informant by clinic staff Examiner s initials: INSTRUCTIONS: Indicate how well each statement describes the subject s CURRENT behavior. There are no right or wrong answers; we just want to get your impression of how you think the subject typically behaves. If you have questions about how to complete this questionnaire, please ask a staff member, and they will be happy to help you. Strongly Disagree (1) Disagree (2) Agree (3) Strongly Agree (4) 1. If the subject thinks something unpleasant is going to happen, he/she usually gets pretty worked up The subject worries about making mistakes Criticism or scolding hurts the subject quite a bit The subject feels pretty worried or upset when he/she thinks somebody is angry at him/her Even if something bad is about to happen to the subject, he/she rarely experiences fear or nervousness The subject feels worried when he/she thinks he/she has done poorly at something The subject has very few fears compared to his/her friends Copyright 1994 by the American Psychological Association. Adapted with permission. The official citation that should be used in referencing this material is Table 1 (adapted), p. 323, from Carver C. S., & White, T. L. (1994). Behavioral inhibition, behavioral activation, and affective responses to impending reward and punishment: The BIS/BAS Scales. Journal of Personality and Social Psychology, 67(2), doi: / No further reproduction or distribution is permitted without written permission from the American Psychological Association. FOR CLINIC USE ONLY: 8. BIS Total Score (7 28): UDS/FTLD Version 2.0, January 2012 Page 1 of 1

26 Initial Visit Packet NACC Uniform Data Set (UDS) ftld module Form C5F: Interpersonal Reactivity Index 1 Informant Questionnaire INSTRUCTIONS FOR THE CENTER This questionnaire is designed to be completed independently by the informant, who will be describing the subject s current typical behavior. This form may be handed to the informant for completion by him- or herself at any time during the study visit. If the informant asks for clarification of questions, it is acceptable for a qualified psychologist or psychometrist to discuss the questionnaire with him or her. However, if the informant completes this questionnaire collaboratively with the clinician, either face-to-face or via telephone, you must inform NACC of this change in protocol by checking the appropriate box in the gray FOR CLINIC USE ONLY area at the top of the questionnaire. Before the informant leaves, clinic staff should make sure that all questions were completed by the informant (i.e., none was left blank) by discussing the missing item with the informant and encouraging them to provide a response. If this is not done and it is later noticed that some items were missed by the informant, clinic staff should call the informant as soon as possible so that the missing items can be completed by phone. In this case, the questionnaire is not considered to have been completed independently by the informant. In the shaded area at the top of the form, the appropriate response would therefore be, This questionnaire was completed via telephone interview of informant by clinic staff. If there are still missing items, these items should be left blank, and 88 should be entered for the Empathic Concern (EC) Score and Perspective-taking (PT) Score. Reproduced by permission of the author, Mark H. Davis, Ph.D.; further copying or distribution without author s permission is prohibited. Form created as part of the National Alzheimer s Coordinating Center s FTLD Module to the Uniform Data Set, copyright 2013 University of Washington. UDS/FTLD Version 2.0, January 2012

27 Initial Visit Packet NACC Uniform Data Set (UDS) ftld module Form C5F: Interpersonal Reactivity Index 1 Informant Questionnaire FOR CLINIC USE ONLY: Examiner s initials: This questionnaire was completed: 0 Independently by informant, as described in Instructions to the Center 1 Via in-person interview of informant by clinic staff 2 Via telephone interview of informant by clinic staff Please give us some information about yourself: Your sex: 1 Male 2 Female Your date of birth (mm/yyyy): / Relationship to subject: 1 Spouse or spouse equivalent 2 Child 3 Daughter- or son-in-law 4 Parent 5 Sibling 6 Other (other relative, friend, neighbor, paid caregiver) DIRECTIONS: Indicate how well each statement describes the subject s CURRENT behavior. There are no right or wrong answers; we just want to get your impression of how you think the subject typically behaves. If you have questions about how to complete this questionnaire, please ask a staff member, and they will be happy to help you. 1. The subject shows tender, concerned feelings for people less fortunate than him/her. 2. The subject sometimes finds it difficult to see things from the other guy s point of view. 3. Sometimes the subject does NOT feel very sorry for other people when they are having problems. 4. The subject tries to look at everybody s side of a disagreement before he/she makes a decision. Does NOT Describes describe VERY well well Reproduced by permission of the author, Mark H. Davis, Ph.D.; further copying or distribution without author s permission is prohibited. Form created as part of the National Alzheimer s Coordinating Center s FTLD Module to the Uniform Data Set, copyright 2013 University of Washington. UDS/FTLD Version 2.0, January 2012 Page 1 of 2

28 Does NOT describe well Describes VERY well 5. If the subject sees somebody being taken advantage of, the subject feels kind of protective towards him/her. 6. The subject is likely to try to understand others better by imagining how things look from their perspective Other people s misfortunes do NOT usually disturb the subject a great deal If the subject is sure he/she is right about something, he/she doesn t waste much time listening to other people s arguments If the subject sees someone being treated unfairly, the subject doesn t feel much pity for him/her. 10. The subject is often quite touched by things he/she sees happen. 11. The subject believes that there are two sides to every question and tries to look at both of them. 12. I would describe the subject as a pretty soft-hearted person. 13. If the subject is upset at someone, the subject usually tries to put him/herself in the other person s shoes for a while. 14. Before criticizing me, the subject is likely to imagine how he/she would feel if he/she were in my place. 1 Davis MH. Measuring individual differences in empathy: evidence for a multidimensional approach. J Pers Soc Psychol 1983; 44(1): FOR CLINIC USE ONLY: 15. Empathic Concern Score (EC) (7 35): 16. Perspective-taking Score (PT) (7 35): Reproduced by permission of the author, Mark H. Davis, Ph.D.; further copying or distribution without author s permission is prohibited. Form created as part of the National Alzheimer s Coordinating Center s FTLD Module to the Uniform Data Set, copyright 2013 University of Washington. UDS/FTLD Version 2.0, January 2012 Page 2 of 2